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Multimodality Imaging of Fabry Disease Cardiomyopathy Complicated With Coronary Vasospasm
Tomohiro Tahara1, Eisuke Usui1, Masahiro Hada1
1Department of Cardiovascular Medicine, Tsuchiura Kyodo General Hospital, Tokyo, Japan.
Insights
A man with angina experienced coronary vasospasm and was diagnosed with Fabry disease. Myocardial fibrosis was detected, possibly due to ischemia or microvascular dysfunction.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Coronary vasospasm can lead to myocardial ischemia and fibrosis.
- Fabry disease is a rare genetic disorder affecting multiple organs, including the heart.
- Myocardial fibrosis is a significant risk factor for adverse cardiac events.
Purpose of the Study:
- To investigate the potential link between coronary vasospasm, Fabry disease, and myocardial fibrosis.
- To evaluate the utility of computed tomography (CT) in assessing cardiac changes in patients with these conditions.
Main Methods:
- Case report of a 41-year-old male patient.
- Diagnosis of coronary vasospasm and Fabry disease (confirmed by low serum α-galactosidase A activity).
- Cardiac imaging using computed tomography (CT) to assess extracellular volume.
Main Results:
- The patient presented with resting angina.
- High CT-derived extracellular volume was observed in the left ventricle's apical inferior wall.
- Findings suggest myocardial fibrosis potentially related to vasospasm-induced ischemia and/or microvascular dysfunction.
Conclusions:
- Coronary vasospasm and Fabry disease can coexist and contribute to myocardial fibrosis.
- CT-derived extracellular volume may serve as a marker for myocardial fibrosis in such cases.
- Early diagnosis and management are crucial for patients with coronary vasospasm and Fabry disease to prevent cardiac complications.
Abstract:
A 41-year-old man with resting angina was diagnosed with a coronary vasospasm and subsequently with Fabry disease exhibiting low serum α-galactosidase A activity. High computed tomography (CT)-derived extracellular volume was detected in the apical inferior wall of the left ventricle suggesting myocardial fibrosis, potentially from vasospasm-related ischemia and/or microvascular dysfunction.
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